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      Adrenocorticotropin Receptor and Adrenal Disorders

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          Abstract

          The ACTH receptor is the shortest G-protein-coupled receptor to date and consists of 297 residues with two putative glycosylation sites at the extracellular N terminus. In vitro studies have demonstrated upregulation of the ACTH receptor by its own ligand and by angiotensin II. Inactivating mutations of the ACTH receptor lead to the familial glucocorticoid deficiency (FGD) syndrome, a rare recessive autosomal disorder characterized by degeneration of the zona fasciculata/reticularis and unresponsiveness to exogenous ACTH. Interestingly, ACTH receptor mutations are not present in all patients with FGD and also not in the closely related ‘triple A’ syndrome indicating that other mechanisms of ACTH resistance are still to be elucidated. Despite an extensive search, no activating ACTH receptor mutations have been found in adrenal tumors, excluding the ACTH receptor as a relevant oncogene for adrenal tumorigenesis. However, the ACTH receptor may play a role as a differentiation factor, as loss of heterozygosity for the ACTH receptor in adrenal tumors seems to be associated with an undifferentiated phenotype. ACTH receptor mRNA expression in benign adrenal tumors is strongly related to the expression of P-450 side chain cleavage enzyme mRNA indicating a close regulative relationship. However, this correlation is disrupted in adrenal carcinomas, an observation which may help in the difficult differential diagnosis between benign and malignant tumors. Surprisingly, the highest ACTH receptor mRNA expression was found in aldosteronomas, while it is low in non-functioning adenomas and carcinomas. No correlation between ACTH receptor mRNA expression and circulating ACTH levels has been found in patients with adrenal disorders casting doubts on the physiological significance of ACTH receptor upregulation by its own ligand in vivo.

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          Author and article information

          Journal
          HRE
          Horm Res Paediatr
          10.1159/issn.1663-2818
          Hormone Research in Paediatrics
          S. Karger AG
          978-3-8055-6504-2
          978-3-318-01928-5
          1663-2818
          1663-2826
          1997
          1997
          09 December 2008
          : 47
          : 4-6
          : 273-278
          Affiliations
          Schwerpunkt Endokrinologie, Medizinische Universitätsklinik, Würzburg, Deutschland
          Article
          185476 Horm Res 1997;47:273–278
          10.1159/000185476
          9167964
          © 1997 S. Karger AG, Basel

          Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

          Page count
          Pages: 6
          Categories
          Recent Progress on the Molecular Aspects of Endocrine Tumors: Clinical Implications

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